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Congress: ECR25
Poster Number: C-14946
Type: Poster: EPOS Radiologist (educational)
Authorblock: L. Nicolosi, F. Tiralongo, C. Ini, D. Grippaldi, E. Ferraro, E. David, P. V. Foti, S. Palmucci, A. Basile; Catania/IT
Disclosures:
Lorena Nicolosi: Nothing to disclose
Francesco Tiralongo: Nothing to disclose
Corrado Ini: Nothing to disclose
Daniele Grippaldi: Nothing to disclose
Elisa Ferraro: Nothing to disclose
Emanuele David: Nothing to disclose
Pietro Valerio Foti: Nothing to disclose
Stefano Palmucci: Nothing to disclose
Antonio Basile: Nothing to disclose
Keywords: Neuroradiology brain, CT, MR, Education, Arteriovenous malformations
Findings and procedure details

DEVELOPMENTAL VENOUS ANOMALIES

Developmental venous anomalies (DVAs), also known as venous angiomas, are the most common cerebral vascular malformations, occurring in 7.46% of the population. DVAs are congenital lesions, characterized by a “caput medusae” or a “palm-tree” configuration of medullary veins draining into a transcortical or subependymal collecting vein of the brain.(Fig.2)

Fig 2: Coronal 3D T1-W GRE post-contrast image shows a vascular structure in the left basal ganglia that drains into subependymal veins of the anterior horn of the left lateral ventricle (arrowhead) (A). Post-contrast axial 2D T1-W SE image of the same patient displays a typical caput medusae appearance of multiple medullary veins collecting on the draining vessels within the lateral ventricle (white arrow) (B).
The most frequent localization is supratentorial, especially in the frontal lobe, followed by the parietal lobe and basal ganglia. DVA generally is an isolated, benign, and asymptomatic condition, sporadically observed on MRI or CT brain scans.[7] However, they can occasionally manifest with complications such as venous infarction, hydrocephalus, or symptomatic hemorrhage, especially when DVA is associated with cavernous malformation.[3] MRI represents the best imaging modality to depict DVA, best visualized with T1-W contrast-enhanced sequences. They present as multiple and linear vascular structures, with a radial course and a typical “caput medusae” appearance, confluent in superficial draining veins.(Fig.3)
Fig 3: Sagittal 3D T1-W post-contrast imaging shows an intraparenchymal venous anomaly (A,B,C), on the right cerebellar hemisphere, draining on the homolateral transverse sinus (dotted arrow)(D). Coronal 3D T1-W post-contrast sequence shows the typical palm-tree aspect of a DVA (arrowhead).
SWI, with phase and magnitude phases, is essential to emphasize the presence of DVA due to the paramagnetic properties of deoxyhemoglobin on anomalous dilated vessels. SWI sequences are also essential for identifying associated lesions, such as cavernomas.[8]

CEREBRAL CAVERNOUS MALFORMATIONS

Cavernomas, or cerebral cavernous malformations (CCMs), are endothelium-lined vascular hamartomas with a mulberry-like aspect, characterized by clusters of abnormally dilated blood vessels. These lesions are prone to hemorrhage due to their vascular fragile structure. Found in 0.4-0.5% of the population, cavernomas may occur sporadically or as part of a familial syndrome, often presenting with multiple lesions. Symptoms range from seizures and focal neurologic deficits to intracranial hemorrhage, which is easily displayed on CT scans.[9] MRI is the gold standard for diagnosis. Pathognomonic features are the “popcorn” or “mulberry” appearance, with a hypointense hemosiderin ring on T2-weighted and SWI sequences, and the absence of contrast enhancement on MRI or CT.(Fig.4)

Fig 4: Patient with incidental findings of cerebral cavernous malformation. Axial T1-W SE sequence shows a hyperintense spot with a hypointense halo (white arrow) (A). Axial T2-W TSE image (B) and axial 3D SWI (C) best capture CM as a mixed-intensity signal (white arrow) with blooming artifacts due to hemosiderin content (white asterisk).
Due to blood-brain barrier disruption, CM is often characterized by thrombosis, calcifications, and hemosiderin deposits, which are sensitively evaluated on T2*-GRE and SWI sequences on MRI.[10] The imaging presentation of these lesions ranges according to Zabramski classification, which differs CCMs into four types, as shown in Table 1
Table 1: Zabramski’s classification of cavernous malformations proposed in 1994.
. Type 1 CCM is characterized by hyperintense lesions on T1-W images and hypo- or hyperintense signals on T2-W, reflecting subacute hemorrhage. Type 2 is the most frequent, with a typical irregular shape and mixed signal intensity on T1 and T2-W sequences, with signal loss on T2* GRE or SWI. Type 3 presents hypointense to isointense core on T1-W and hypointense core on T2, typical of subacute hemorrhage. Type 4 CCMs are commonly defined as microhemorrhages, with isointense signal to brain parenchyma on T1 and T2-W images, recognizable exclusively on T2* GRE or SWI as hypointense dots with blooming artifacts.(Fig.5)
Fig 5: Axial 2D T2* GRE enhances the presence of a hypointense spot lesion with blooming artifacts on the left temporal lobe (white arrow) (A), merely visible on axial 2D T2-W TSE and axial 3D T1-W GRE (dotted arrows) (B,C).
Recently type V CCM has been added, characterized by extralesional hemorrhage.[11](Fig.6)
Fig 6: Illustration of Zabramski’s classification, with the additional category of type V CCM characterized by cavernous malformation associated with extralesional hemorrhage.
Management varies from observation to surgical resection, depending on symptom severity and lesion location.[2][12]

CAPILLARY TELANGIECTASIAS

Capillary telangiectasias are benign vascular malformations composed of thin-walled ectatic capillaries interspersed with normal brain parenchyma. These lesions are usually asymptomatic and discovered incidentally. Hemorrhage is rare, but the presence of associated lesions should prompt further investigation. Typically located in the pons, these lesions are best detected with contrast-enhanced MRI, appearing as faintly enhancing areas without mass effect or edema. SWI sequences can demonstrate signal loss due to slow-flowing blood.[13](Fig.7)

Fig 7: Axial 2D T2*-W GRE image shows a small hypointensity lesion of the median region of the pons (dotted white arrow). Axial 3D T1-W GRE post-contrast image (C) displays a focal contrast enhancement with a “spot in a dot” aspect (arrowhead) barely evident on axial 3D T1-W GRE pre-contrast imaging (white arrow) (B), features of pontine capillary telangiectasia.

SINUS PERICRANII

Sinus pericranii involves the abnormal connection between intracranial sinuses and epicranial veins through subgaleal veins that might appear notably enlarged and varicose. SP typically presents as a soft, compressible scalp mass that varies in size with changes in intracranial pressure. While mostly asymptomatic, complications such as thrombosis or hemorrhage may occur. MRI and CT venography are crucial for diagnosis, demonstrating extracranial venous structures communicating with intracranial sinuses through a skull defect.(Fig.8)

Fig 8: Sagittal CT venography image with maximum intensity projection (MIP) reconstruction shows some anomalous dilated vessels (white asterisk) within subcutaneous scalp tissues (A). Axial from a CT with venous phase demonstrates the anomalous connection between the subgaleal vein with the superior sagittal sinus (black arrow)(B) within a defect of the parietal bone, best validated on thin-slices bone reconstruction (white arrow)(C).
Most cases are managed conservatively, but surgical resection may be indicated for cosmetic concerns or symptomatic lesions.[14]

MIXED VASCULAR ANOMALIES

Due to their shared pathogenesis or causal-evolutionary relationship, these conditions frequently coexist as “mixed vascular anomalies” (MVAs). The first and most frequently described combination of MVAs in Literature is DVA with CCM, which proposes theories of their coexistence.(Fig.9)

Fig 9: Coronal 3D T1-W GRE sequence shows a linear enhancement on the left nucleo-capsular region (white arrow) (A), that on 3D SWI image is characterized by magnetic susceptibility artifacts (black arrow) (B) suggestive of a DVA. DVA appears near the cavernous malformation (thick white arrow) as mixed vascular anomalies.
Continue microhemorrhages within DVA seem to induce the release of pro-angiogenetic factors, thereby promoting CM formation. The presence of MVAs is clinically significant as it might heighten the risk of bleeding complications.[7]
GALLERY